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Literature summary extracted from

  • O'Reilly, L.; Bross, P.; Corydon, T.J.; Olpin, S.E.; Hansen, J.; Kenney, J.M.; McCandless, S.E.; Frazier, D.M.; Winter, V.; Gregersen, N.; Engel, P.C.; Andresen, B.S.
    The Y42H mutation in medium-chain acyl-CoA dehydrogenase, which is prevalent in babies identified by MS/MS-based newborn screening, is temperature sensitive (2004), Eur. J. Biochem., 271, 4053-4063.
    View publication on PubMed

Protein Variants

EC Number Protein Variants Comment Organism
1.3.8.7 K304E maximal velocity of the mutant protein is only one third of the wild-type value. 16% higher activity with the natural electron acceptor, electron transferring flavoprotein, than the wild-type enzyme. Decreased stability at temperatures above 42°C compared to wild-type enzyme Rattus norvegicus
1.3.8.7 Y42H mutant enzyme has approximately the same maximal velocity as the wild-type enzyme, the KM-value for octanoyl-CoA is 1.6fold higher than the wild-type value. 7% higher activity with the natural electron acceptor, electron transferring flavoprotein, than the wild-type enzyme. Decreased stability at temperatures above 42°C compared to wild-type enzyme Rattus norvegicus

KM Value [mM]

EC Number KM Value [mM] KM Value Maximum [mM] Substrate Comment Organism Structure
1.3.8.7 0.0037
-
 octanoyl-CoA wild-type enzyme Rattus norvegicus
1.3.8.7 0.0059
-
 octanoyl-CoA mutant enzyme K304E Rattus norvegicus

Localization

EC Number Localization Comment Organism GeneOntology No. Textmining
1.3.8.7 mitochondrion
-
 Rattus norvegicus 5739
-

Natural Substrates/ Products (Substrates)

EC Number Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
1.3.8.7 additional information Rattus norvegicus a Y42H mutation is frequently found in babies identified by newborn screening with MS/MS. Y42H is a temperature sensitive mutation, which is mild at low temperatures, but may have deleterious effects at increased temperatures ?
-
 ?
1.3.8.7 octanoyl-CoA + electron transferring flavoprotein Rattus norvegicus
-
 2-octenoyl-CoA + reduced electron transferring flavoprotein
-
 ?

Organism

EC Number Organism UniProt Comment Textmining
1.3.8.7 Rattus norvegicus
-
-
-

Purification (Commentary)

EC Number Purification (Comment) Organism
1.3.8.7
-
 Rattus norvegicus

Source Tissue

EC Number Source Tissue Comment Organism Textmining
1.3.8.7 liver
-
 Rattus norvegicus
-

Substrates and Products (Substrate)

EC Number Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
1.3.8.7 additional information a Y42H mutation is frequently found in babies identified by newborn screening with MS/MS. Y42H is a temperature sensitive mutation, which is mild at low temperatures, but may have deleterious effects at increased temperatures Rattus norvegicus ?
-
 ?
1.3.8.7 octanoyl-CoA + electron transferring flavoprotein
-
 Rattus norvegicus 2-octenoyl-CoA + reduced electron transferring flavoprotein
-
 ?
1.3.8.7 octanoyl-CoA + ferricenium
-
 Rattus norvegicus 2-octenoyl-CoA + reduced acceptor
-
 ?

Synonyms

EC Number Synonyms Comment Organism
1.3.8.7 MCAD
-
 Rattus norvegicus
1.3.8.7 medium-chain acyl-CoA dehydrogenase
-
 Rattus norvegicus

Temperature Stability [°C]

EC Number Temperature Stability Minimum [°C] Temperature Stability Maximum [°C] Comment Organism
1.3.8.7 42
-
 decreased stability of mutant enzymes Y42H and K304E above 42°C compared to wild-type enzyme Rattus norvegicus